Case for Diagnosis
Erythematous-Violaceous Plaques on the Limbs of a Patient with Rheumatoid Arthritis and Colon Cancer
Placas eritematovioláceas en extremidades de paciente con artritis reumatoide y cáncer de colon
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She presented at the emergency room with erythematous lesions on the limbs and a fever of 24 hours’ duration.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">Physical examination revealed erythematous macules and plaques that were very painful on palpation on the inner aspect of the right foot, the palm of the left hand, and the abdomen and thighs (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">A skin biopsy was taken from the affected area of the right foot.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Histopathology</span><p id="par0020" class="elsevierStylePara elsevierViewall">Histologic examination revealed marked dilatation of vessels of the superficial and deep dermis, the lumina of which contained large mononuclear cells with broad granular, eosinophilic cytoplasm and little atypia (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). Immunohistochemistry revealed that these cells were positive for CD68, CD45, and CD43, and negative for CD20, CD3, cytokeratins 7 and 20, CD31, and factor VIII. The endothelium was positive for D2-40. No neutrophilic elements or markers of malignancy were observed.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Additional Tests</span><p id="par0025" class="elsevierStylePara elsevierViewall">Analyses performed in the emergency room revealed a normal blood count and elevated levels of fibrinogen and C-reactive protein. All other biochemical and coagulation parameters were normal. Blood, urine, and skin biopsy cultures were all negative.</p><p id="par0030" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">What Is Your Diagnosis?</span></p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Diagnosis</span><p id="par0035" class="elsevierStylePara elsevierViewall">Intralymphatic histiocytosis.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Clinical Course and Treatment</span><p id="par0040" class="elsevierStylePara elsevierViewall">The patient was treated empirically with amoxicillin-clavulanate (875<span class="elsevierStyleHsp" style=""></span>mg/125<span class="elsevierStyleHsp" style=""></span>mL) every 8<span class="elsevierStyleHsp" style=""></span>hours, and treatment with prednisone (0.5<span class="elsevierStyleHsp" style=""></span>mg /kg/day) was initiated 5 days later. Within 2 weeks both the fever and skin lesions had resolved.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Comment</span><p id="par0045" class="elsevierStylePara elsevierViewall">Intralymphatic histiocytosis is a rare condition with diverse clinical manifestations but characteristic histology and immunohistochemistry. Most reported cases are associated with chronic inflammatory processes, particularly rheumatoid arthritis, and less frequently joint replacement and tumors such as breast cancer, Merkel cell carcinoma, and colonic neoplasia.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–5</span></a> While the etiology and pathogenesis of this condition are unknown, its association with these diseases suggests that it could be due to lymphatic stasis and dilation of lymphatic vessels, in which the persistent presence of antigens stimulates the aggregation and proliferation of histiocytes.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">The most common clinical presentation is characterized by poorly defined erythematous plaques, mainly on the limbs, sometimes in association with livedo reticularis or superficial papules, vesicles, or nodules.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> Fever has not been reported in any of the cases described to date, but is commonly observed in immunosuppressed patients undergoing chemotherapy. Similarly, local pain has not been previously reported, and may be due to the almost total vascular occlusion observed in our patient.</p><p id="par0055" class="elsevierStylePara elsevierViewall">Histologically, intralymphatic histiocytosis is characterized by dilatation of vessels in the dermis and the presence of large mononuclear cells with granular eosinophilic cytoplasm in the vascular lumina. Immunohistochemical techniques are fundamental to confirm that the dilated vessels are lymphatic vessels (positive for D2-40, Lyve-1, and Prox-1), and that the cells occluding the vessels are histiocytes (CD68<span class="elsevierStyleSup">+</span>).<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">The lesions usually follow a benign course and tend to resolve spontaneously, although in many cases a chronic course is observed. Multiple treatments have been used,<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> including radiation therapy, topical corticosteroids, cyclophosphamide, pentoxifylline, infliximab,<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> amoxicillin, and aspirin.</p><p id="par0065" class="elsevierStylePara elsevierViewall">Histologic differential diagnosis should include intravascular lymphoma and reactive angioendotheliomatosis.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> Intravascular lymphoma is a rare and aggressive subtype of large B-cell lymphoma, which mainly affects the vessels of the skin and central nervous system and is associated with a poor prognosis. Histologic examination and immunohistochemistry can confirm that the dilated vessels are blood vessels and the cells they contain are lymphocytes, usually B-lymphocytes and hence CD20<span class="elsevierStyleSup">+</span> and CD79a<span class="elsevierStyleSup">+</span>. Angioendotheliomatosis is a benign reactive process characterized by the proliferation of endothelial cells that obliterate blood vessels, and is associated with infectious diseases such as tuberculosis, hematologic neoplasms, leukocytoclastic vasculitis, occlusive vascular disorders, and rheumatoid arthritis The blood vessels are dilated and are not reactive to D2-40, Lyve-1, or Prox-1, while endothelial cells exhibit CD31, CD34 and factor VIII immunoreactivity.</p><p id="par0070" class="elsevierStylePara elsevierViewall">Other conditions that present with histiocytes are Rosai-Dorfman disease (with emperipolesis and S-100 immunoreactivity), Melkersson-Rosental syndrome, sclerosing lymphangitis of the penis, and granulomatous lymphangitis of the penis and scrotum.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Furthermore, in cases where the vascular lumina are occluded by abundant cellular elements, metastasis of solid carcinomas and leukemias must be ruled out.</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Conflict of Interest</span><p id="par0075" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:10 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Medical History" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Physical Examination" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Histopathology" ] 3 => array:2 [ "identificador" => "sec0020" "titulo" => "Additional Tests" ] 4 => array:2 [ "identificador" => "sec0025" "titulo" => "Diagnosis" ] 5 => array:2 [ "identificador" => "sec0030" "titulo" => "Clinical Course and Treatment" ] 6 => array:2 [ "identificador" => "sec0035" "titulo" => "Comment" ] 7 => array:2 [ "identificador" => "sec0040" "titulo" => "Conflict of Interest" ] 8 => array:2 [ "identificador" => "xack35234" "titulo" => "Acknowledgments" ] 9 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara">Please cite this article as: P. Borregón, J.A. Avilés, V. Parra. Placas eritematovioláceas en extremidades de paciente con arthritis reumatoide y cáncer de colon. Actas Dermosifiliogr. 2012;103:543-4.</p>" ] ] "multimedia" => array:2 [ 0 => array:6 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 634 "Ancho" => 900 "Tamanyo" => 72866 ] ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1115 "Ancho" => 1585 "Tamanyo" => 510522 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Hematoxylin-eosin, original magnification ×40; Hematoxylin-eosin, original magnification ×20; CD68, original magnification ×20.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Intralymphatichistiocytosis. A clinicopathologic study of 16 cases" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "L. Requena" 1 => "L. El-Shabrawi-Caelen" 2 => "S.N. Walsh" 3 => "S. Segura" 4 => "M. Ziemer" 5 => "M.A. Hurt" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1097/DAD.0b013e3181986cc2" "Revista" => array:6 [ "tituloSerie" => "Am J Dermatopathol" "fecha" => "2009" "volumen" => "31" "paginaInicial" => "140" "paginaFinal" => "151" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/19318799" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0010" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Histiocitosis intralinfática y neoplasia de colon" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "B. Echeverría-García" 1 => "R. Botella-Estrada" 2 => "C. Requena" 3 => "C. 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Cheng" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/j.1365-2230.2009.03255.x" "Revista" => array:6 [ "tituloSerie" => "Clin Exp Dermatol" "fecha" => "2009" "volumen" => "34" "paginaInicial" => "e302" "paginaFinal" => "e303" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/19456788" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0020" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Erythematous patch overlying a swollen knee" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "Y.E. Chiu" 1 => "J.E. Maloney" 2 => "C. Bengana" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1001/archdermatol.2010.215-a" "Revista" => array:6 [ "tituloSerie" => "Arch Dermatol" "fecha" => "2010" "volumen" => "146" "paginaInicial" => "1037" "paginaFinal" => "1042" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/20855711" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0025" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Effectiveness of infliximab for intralymphatic histiocytosis with rheumatoid arthritis" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "M. Sakaguchi" 1 => "H. Nagai" 2 => "G. Tsuji" 3 => "A. Morinobu" 4 => "S. Kumagai" 5 => "C. Nishigori" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1001/archdermatol.2010.407" "Revista" => array:6 [ "tituloSerie" => "Arch Dermatol" "fecha" => "2011" "volumen" => "147" "paginaInicial" => "131" "paginaFinal" => "133" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21242414" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] "agradecimientos" => array:1 [ 0 => array:3 [ "identificador" => "xack35234" "titulo" => "Acknowledgments" "texto" => "<p id="par0080" class="elsevierStylePara elsevierViewall">We are sincerely grateful to the department of pathology, whose input is often essential in diagnoses.</p>" ] ] ] "idiomaDefecto" => "en" "url" => "/15782190/0000010300000006/v1_201304241313/S1578219012002089/v1_201304241313/en/main.assets" "Apartado" => array:4 [ "identificador" => "6154" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Case for Diagnosis" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/15782190/0000010300000006/v1_201304241313/S1578219012002089/v1_201304241313/en/main.pdf?idApp=UINPBA000044&text.app=https://actasdermo.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1578219012002089?idApp=UINPBA000044" ]
| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 6 | 14 | 20 |
| 2024 Octubre | 76 | 74 | 150 |
| 2024 Septiembre | 124 | 37 | 161 |
| 2024 Agosto | 122 | 69 | 191 |
| 2024 Julio | 89 | 27 | 116 |
| 2024 Junio | 87 | 29 | 116 |
| 2024 Mayo | 96 | 49 | 145 |
| 2024 Abril | 68 | 35 | 103 |
| 2024 Marzo | 84 | 45 | 129 |
| 2024 Febrero | 106 | 38 | 144 |
| 2024 Enero | 57 | 29 | 86 |
| 2023 Diciembre | 42 | 28 | 70 |
| 2023 Noviembre | 85 | 32 | 117 |
| 2023 Octubre | 76 | 20 | 96 |
| 2023 Septiembre | 62 | 32 | 94 |
| 2023 Agosto | 54 | 20 | 74 |
| 2023 Julio | 55 | 37 | 92 |
| 2023 Junio | 66 | 25 | 91 |
| 2023 Mayo | 78 | 26 | 104 |
| 2023 Abril | 55 | 24 | 79 |
| 2023 Marzo | 54 | 29 | 83 |
| 2023 Febrero | 57 | 23 | 80 |
| 2023 Enero | 64 | 31 | 95 |
| 2022 Diciembre | 77 | 45 | 122 |
| 2022 Noviembre | 38 | 20 | 58 |
| 2022 Octubre | 39 | 18 | 57 |
| 2022 Septiembre | 66 | 39 | 105 |
| 2022 Agosto | 75 | 34 | 109 |
| 2022 Julio | 57 | 31 | 88 |
| 2022 Junio | 39 | 26 | 65 |
| 2022 Mayo | 70 | 28 | 98 |
| 2022 Abril | 55 | 43 | 98 |
| 2022 Marzo | 51 | 44 | 95 |
| 2022 Febrero | 44 | 27 | 71 |
| 2022 Enero | 44 | 30 | 74 |
| 2021 Diciembre | 40 | 48 | 88 |
| 2021 Noviembre | 47 | 37 | 84 |
| 2021 Octubre | 54 | 48 | 102 |
| 2021 Septiembre | 48 | 40 | 88 |
| 2021 Agosto | 64 | 45 | 109 |
| 2021 Julio | 47 | 36 | 83 |
| 2021 Junio | 41 | 33 | 74 |
| 2021 Mayo | 49 | 32 | 81 |
| 2021 Abril | 80 | 79 | 159 |
| 2021 Marzo | 52 | 31 | 83 |
| 2021 Febrero | 55 | 23 | 78 |
| 2021 Enero | 46 | 21 | 67 |
| 2020 Diciembre | 40 | 14 | 54 |
| 2020 Noviembre | 37 | 16 | 53 |
| 2020 Octubre | 19 | 16 | 35 |
| 2020 Septiembre | 34 | 8 | 42 |
| 2020 Agosto | 29 | 20 | 49 |
| 2020 Julio | 32 | 17 | 49 |
| 2020 Junio | 41 | 32 | 73 |
| 2020 Mayo | 28 | 17 | 45 |
| 2020 Abril | 34 | 21 | 55 |
| 2020 Marzo | 30 | 22 | 52 |
| 2020 Febrero | 10 | 1 | 11 |
| 2020 Enero | 4 | 4 | 8 |
| 2019 Diciembre | 4 | 5 | 9 |
| 2019 Noviembre | 4 | 1 | 5 |
| 2019 Octubre | 0 | 4 | 4 |
| 2019 Septiembre | 3 | 3 | 6 |
| 2019 Agosto | 4 | 2 | 6 |
| 2019 Julio | 4 | 2 | 6 |
| 2019 Junio | 4 | 16 | 20 |
| 2019 Mayo | 4 | 53 | 57 |
| 2019 Abril | 2 | 18 | 20 |
| 2019 Marzo | 0 | 10 | 10 |
| 2019 Febrero | 2 | 3 | 5 |
| 2019 Enero | 1 | 2 | 3 |
| 2018 Diciembre | 0 | 6 | 6 |
| 2018 Noviembre | 1 | 4 | 5 |
| 2018 Octubre | 1 | 0 | 1 |
| 2018 Septiembre | 4 | 0 | 4 |
| 2018 Agosto | 0 | 3 | 3 |
| 2018 Julio | 0 | 2 | 2 |
| 2018 Junio | 0 | 1 | 1 |
| 2018 Mayo | 0 | 3 | 3 |
| 2018 Abril | 0 | 1 | 1 |
| 2018 Marzo | 2 | 3 | 5 |
| 2018 Febrero | 105 | 16 | 121 |
| 2018 Enero | 88 | 3 | 91 |
| 2017 Diciembre | 72 | 7 | 79 |
| 2017 Noviembre | 48 | 11 | 59 |
| 2017 Octubre | 49 | 6 | 55 |
| 2017 Septiembre | 59 | 6 | 65 |
| 2017 Agosto | 74 | 14 | 88 |
| 2017 Julio | 76 | 12 | 88 |
| 2017 Junio | 80 | 18 | 98 |
| 2017 Mayo | 82 | 18 | 100 |
| 2017 Abril | 70 | 6 | 76 |
| 2017 Marzo | 47 | 38 | 85 |
| 2017 Febrero | 32 | 8 | 40 |
| 2017 Enero | 38 | 8 | 46 |
| 2016 Diciembre | 47 | 6 | 53 |
| 2016 Noviembre | 65 | 28 | 93 |
| 2016 Octubre | 74 | 17 | 91 |
| 2016 Septiembre | 107 | 8 | 115 |
| 2016 Agosto | 74 | 10 | 84 |
| 2016 Julio | 53 | 25 | 78 |
| 2016 Junio | 15 | 17 | 32 |
| 2016 Mayo | 10 | 10 | 20 |
| 2016 Abril | 10 | 7 | 17 |
| 2016 Marzo | 5 | 5 | 10 |
| 2016 Febrero | 6 | 3 | 9 |
| 2016 Enero | 13 | 1 | 14 |
| 2015 Diciembre | 15 | 1 | 16 |
| 2015 Noviembre | 25 | 2 | 27 |
| 2015 Octubre | 26 | 5 | 31 |
| 2015 Septiembre | 22 | 2 | 24 |
| 2015 Agosto | 10 | 1 | 11 |
| 2015 Julio | 92 | 13 | 105 |
| 2015 Junio | 71 | 10 | 81 |
| 2015 Mayo | 93 | 7 | 100 |
| 2015 Abril | 97 | 7 | 104 |
| 2015 Marzo | 90 | 9 | 99 |
| 2015 Febrero | 69 | 4 | 73 |
| 2015 Enero | 86 | 13 | 99 |
| 2014 Diciembre | 89 | 12 | 101 |
| 2014 Noviembre | 70 | 17 | 87 |
| 2014 Octubre | 82 | 15 | 97 |
| 2014 Septiembre | 64 | 10 | 74 |
| 2014 Agosto | 84 | 16 | 100 |
| 2014 Julio | 86 | 20 | 106 |
| 2014 Junio | 49 | 2 | 51 |
| 2014 Mayo | 54 | 5 | 59 |
| 2014 Abril | 46 | 4 | 50 |
| 2014 Marzo | 39 | 9 | 48 |
| 2014 Febrero | 51 | 5 | 56 |
| 2014 Enero | 39 | 6 | 45 |
| 2013 Diciembre | 49 | 6 | 55 |
| 2013 Noviembre | 33 | 8 | 41 |
| 2013 Octubre | 22 | 2 | 24 |
| 2013 Septiembre | 12 | 3 | 15 |
| 2013 Agosto | 14 | 8 | 22 |
| 2013 Julio | 10 | 24 | 34 |
| 2013 Junio | 14 | 12 | 26 |
| 2013 Mayo | 16 | 8 | 24 |
| 2013 Abril | 22 | 6 | 28 |
| 2013 Marzo | 24 | 8 | 32 |
| 2013 Febrero | 40 | 4 | 44 |
| 2013 Enero | 51 | 5 | 56 |
| 2012 Diciembre | 31 | 14 | 45 |
| 2012 Noviembre | 2 | 0 | 2 |
| 2012 Octubre | 1 | 1 | 2 |
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